Neurodegeneration with brain iron accumulation for stem-cell therapy: Anesthetic management

Abstract

Neurodegeneration with brain iron accumulation (Hallervorden-Spatz Disease) is a rare, slowly progressing disorder characterized by progressive extrapyramidal dysfunction resulting in dementia, dystonia, torticollis, ataxia, kyphoscoliosis, and seizures. In chronic cases, muscle contractures, bony changes, temporomandibular joint, and cervical spine immobility may occur. Due to chronic malnutrition, there is increased the tendency for pathological fractures. Any noxious stimulus (anxiety, pain, light plane of anesthesia) intensifies dystonia. Significant speech disturbances, confusion, disorientation, mental retardation, dementia, and visual loss make communication with these patients a problem. Managing anesthesia in such patients is quite challenging as there is difficulty in positioning, intravenous cannulation, and airway management. We present a case report of a 14-year-old male patient with neurodegeneration with brain iron accumulation who was administered anesthesia for the stem-cell therapy.

Keywords

• Anesthesia
• neurodegeneration with brain iron accumulation
• pantothenate kinase-associated neurodegeneration